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  • Author: Orhideja Stomnaroska x
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Abstract

We report a 10 days old newborn with brachycephaly, midfacial hypoplasia, syndactyly and broad distal phalanx of thumb and big toe. At the 20th gestational weeks an enlargement of the left cerebral ventricle and malformation of the fingers of the hands and toes were noticed on a regular ultrasound examination. The aforementioned malformations were observed at birth and at the age of 11 months. The large fontal was closed; the small one was palpable at the tip of the finger. Brachycephaly was evident with high full forehead, flat occiput, and irregular craniosynostosis especially at the coronal suture. Cutaneous syndactyly was present at both hands (fingers II-V), with almost complete fusion of the second, third and fourth fingers. Distal phalanges of the thumbs were broad as well as distal hallux. There was cutaneous syndactyly of the feet. Mental development at the age of 11 months was normal.

Apert syndrome is a sporadic disorder. Rarely, inheritance is autosomal dominant. Appropriate management includes surgical treatment of the syndactylies, follow up of the eventual airway compromise and hearing difficulties. This is a report of a patient identified as a newborn.

Abstract

Neonatal hypoglycemia (HG) can cause neurologic damage, epilepsy, mental retardation, behavioral and personality disorders and death. The longest the HG lasts and the greatest the glucose nadir the consequences are more pronounced.

Comorbidities are rather important in development of neurological damage. Hypoxemia and ischemia can cause permanent brain damage. Small for gestational age (SGA), large for gestational age (LGA), intrauterine growth restriction, gestational age bellow the 37th week, low Apgar score, sepsis, children whose mothers have toxemia, diabetes or chorioamnionitis are all newborns with increased HG risk.

Comparing 34 patients with NH and 34 children without NH with similar GA, BW, BL, the Apgar score, we found statistically significant differences in motor and mental development using the Griffith scale. Children with neonatal HG fared significantly worse than those without neonatal HG. Therefore, CBG measurements and early recognition of neonatal HG is of significant importance in preventing motor and mental damage in children. A larger and well-balanced cohort of patients followed for a longer period is also necessary to clarify and discern in detail the importance of neonatal HG and other perinatal factors in neurodevelopmental damage.

Abstract

Background and aims: Severe neonatal hypoglycemia (HG) leads to neurologic damage, mental retardation, epilepsy, personality disorders, impaired cardiac performance and muscle weakness. We aimed to assess the clinical characteristics of children with hypoglycemia in a random population of newborns.

Patients, methods and results: We investigated 84 patients (M:F=35:48) born at the University Clinic for Gynecology and Obstetrics in Skopje (hospitalized in the NICU) who were found to have hypoglycemia. In total 89.25% of the babies were premature. The mean birth weight was 1795.95 +/596.08 grams, the mean birth length was 41.92+/- 4.62 cm, while the mean gestational age was 33.05±3.19 weeks. 32 children (38.08%) were very low birth weight (<1500g), 38 (45.22%) were low birth weight (1500-2500g), while there were 8 children (9.52%) appropriate for age BW and no high BW for age patients (>4000 g).

HG duration was 2.42+/-2.41 hours. In the group as a whole, hypoxic-ischemic encephalopathy (HIE) was found in 3 children (3.57%), infections in 22 (26.18%), respiratory distress syndrome (RDS) in 9 patients (10.62%), intracranial haemorrhage in 2 patients (2.38%). There were no inborn errors of metabolism. There were two deaths (2.38%).

Conclusion: Neonatal HG is a significant factor in the overall neonatal mortality. HG can also cause severe invalidity. We found that infections, LBW and low gestational age were most commonly associated with neonatal HG. However the Spearman test showed weak direct correlation, without statistical significance. Neonatal HG requires complex and team interaction of prenatal and postnatal approaches to reduce the incidence of seizures, their consequences and the overall mortality. Special consideration is to be taken in measures that avoid neonatal infections, HIE, LBW and low gestational age. Further studies on a larger population are needed to fully understand and prevent the phenomenon of HG in newborns.

Abstract

Neonatal hypoglycemia (NH) is one of the most common abnormalities encountered in the newborn. Maintaining glucose homeostasis is one of the important physiological events during fetal-to-neonatal transition. Transient low blood glucose concentrations are frequently encountered in the majority of healthy newborns and are the reflections of normal metabolic adaptation processes. Nevertheless, there is a great concern that prolonged or recurrent low blood glucose levels may result in long-term neurological and developmental consequences.

Strikingly, it was demonstrated that the incidence and timing of low glucose concentrations in the groups most at risk for asymptomatic neonatal hypoglycemia, did not find association between repetitive low glucose concentrations and poor neurodevelopmental outcomes. On the contrary, NH due to hyperinsulinism is strongly associated with brain injury.

Fundamental issue of great professional controversy is concerning the best manner to manage asymptomatic newborns NH. Both, overtreating NH and undertreating NH are poles with significant potential disadvantages.

Therefore, NH is one of the most important issues in the day-to-day practice. This article appraises the critical questions of definition (widely accepted blood glucose concentration: < 2.6 mmol/l or 47 mg/dl), follow-up ad management of NH.

Abstract

Aim: Severe neonatal hypoglycaemia (HG) leads to neurologic damage, mental retardation, epilepsy, impaired cardiac performance and muscle weakness. The aim was to assess the frequency and severity of HG in a population of newborns.

Patients and methods: We investigated 739 patients with neonatal hypoglycaemia (HG) (M:F=370:369) born at the University Clinic for Gynaecology and Obstetritics in Skopje in the period 2014-2016 and treated at the neonatal intensive care unit (NICU). 1416 babies were treated in the same period in NICU, and HG was observed in 52.18%. The birth weight was dominated by children with low birth weight: very low birth weight (VLBW)(<1500g) 253 children, (34,23%), low birth weight (1500-2500g) 402 (54.39%), appropriate for gestational age (AGA) 78(10.55%), and high birth weight (>4000g) 6 babies (0.81%). The gestational age was also dominated by children with low gestational age: gestational week (GW) 20-25 four children (0.54%), 26-30 GW 133 babies (17.99%), 31-35 GW472 (63.87%), and 36-40 GW130 neonates (17.59 %).

241 mothers (32.61%) have had an infection during pregnancy, 82 preeclampsia or eclampsia (11.09%), 20 diabetes mellitus (2.70%), 78 placental situations (placenta previa, abruption) (10.55%). In this study 47 babies (6.35%) with HG and co-morbidities died.

There was a significant positive correlation between HG birth weight (p<0.01), gestational age (p<0.05), and the lowest Apgar score (p<0.01). Neonatal deaths were significantly correlated with GA (р>0,01), co-morbidities of the mothers (р>0,05) but not with the birth weight (р>0,05). In contrast, a significant positive correlation was found between convulsions and body weight (р<0.05). The lowest Apgar score was positively correlated with the gestational age (0.01), but not with the birth weight (0.05).

Conclusion: Low birth weight, low gestational age, maternal risk factors, hypoxic-ischemic encephalopathy and neonatal infections are associated with HG and are a significant factor in overall neonatal mortality. Those results indicate that diminishing the frequency of the neonatal HG and the rates of neonatal mortality requires complex interaction of prenatal and postnatal interventions.